State of the Art, Current Perspectives, and Controversies of Budd-Chiari Syndrome: A Review

Paschalis Gavriilidis, Gabriele Marangoni, Jawad Ahmad, Daniel Azoulay


Background: Budd-Chiari syndrome (BCS) is an eponym that includes a group of conditions characterized by partial or complete hepatic venous tract outflow obstruction, and the site of obstruction may involve one or more hepatic veins, inferior vena cava, or the right atrium. The classification of BCS is based on etiology, site of obstruction, and duration. Its etiology is very heterogeneous; in particular, hepatic vein thrombosis is the most common type of obstruction and myeloproliferative disorder, the most common thrombophilic disorder, in the West. In Asian countries, the type of obstruction, thrombophilic disorders, clinical features, and treatment strategies vary widely from region to region. Although the cause can be identified in 90% of patients with the help of gene mutation testing, BCS remains under-recognized in many countries. A higher prevalence of acute cases has been reported in the West than in the East. This global and regional heterogeneity raises several challenges regarding the evaluation, management strategy, and individualized approach of BCS. This study aimed to conduct a systematic review of BCS to elucidate treatment strategy options.

Methods: PubMed, Embase, Cochrane Library, and Google Scholar databases were searched systematically.

Results: Sixty-nine pertinent articles were retrieved and included in the present study.

Conclusions: Further research on the following three topics would help define individualized treatment strategies. The first is a better understanding of the molecular pathways underlying the thrombophilic conditions implicated in the pathogenesis of BCS. The second is the role of the genotype and gene mutations in the determination of coagulation status of patients with BCS. The third is the definition of clear criteria and development of a common prognostic index to risk stratify the patients at presentation and consequently detect candidates for invasive therapies.

J Clin Med Res. 2022;14(4):147-157


Budd-Chiari syndrome; Hepatic outflow obstruction; Hepatic vein thrombosis; Vascular liver diseases; Transjugular intrahepatic portosystemic shunt; Liver transplantation; Systematic review

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